(遗传性血管性水肿)。

E W Nielsen, H Skjeflo
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引用次数: 0

摘要

遗传性血管性水肿是一种罕见的疾病,以常染色体显性性状传播。该病通常表现为急性皮下或粘膜下肿胀和持续2至5天的腹痛。当局限于头部或喉咙时,可能会发生危及生命的喉部水肿。该疾病是由活性C1抑制剂的减少引起的。水肿不是由于过敏反应和药物治疗是很少或没有价值。当发作频繁时,应在拔牙、喉插管、大手术(尤其是口咽部手术)和分娩前给予丙炔醇类药物。急性发作时静脉注射C1抑制剂浓缩液治疗效果最好。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Hereditary angioedema].

Hereditary angioedema is a rare disease, transmitted as an autosomal dominant trait. The disease usually manifests as acute subcutaneous or submucosal swellings and abdominal pain of two to five days duration. When localised to head or throat life-threatening edema of the larynx may occur. The disease is caused by reduced amount of active C1 inhibitor. The edema is not due to an allergic reaction and medication for such is of little or no value. Profylactic medication may be given when the attacks are frequent and ought to be given before dental extractions, intubation of the larynx, major surgery especially of the oropharynx, and births. The acute attack is best treated by intravenous injection of C1 inhibitor concentrate.

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